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Human Chiari and the Companion Cavalier Club Talk

Most of you know we recently had a health talk in Wimbledon. One of the speakers is a Chiari sufferer, this lady (Lynn) travelled all the way from Yorkshire and describes her struggle with daily life. I spent a lot of time with Lynn and was able to gain a good basic understanding of the pain and discomfort our dogs are feeling.

This has changed my view of my dogs.

The majority of dogs that have sm will also have cm.

Thankfully there was a lot of media coverage from PDE that brought sm to the attention of the public. Therefore some pet owners were able to obtain the correct treatment to bring sm pain under control. The way I understand it, sm causes pain because of nerve pain caused by a syrix or syrinxes.

Very early on my Dog Molly was diagnosed with sm, her pain was finally controlled with the correct medication.

My other dog Dougall did not show any signs of pain or discomfort, despite this I had him mri'd. Dougall was diagnosed with cm. Eventually it became apparent he was suffering from head/neck and back pain and has always been the focus of our attention.

In the background, Molly has remained stable and not shown any signs of extreme pain. However, she has always remained quiet, doesn't particulary play and is certainly exercise intolerant.

I always looked at Dougall as my CM Dog and Molly as my SM Dog, (by the way, Molly has never scratched or shown any of the classic signs!)

I never took Molly's CM problems into consideration, I only ever thought of her SM.

Cavaliers very quickly learn to adapt to pain, they are more tolerant than most dogs because of their high levels of serotonin. How much pain are they in when they finally show us they are suffering?

I believe, I have made a mistake with Molly!

Lynn has given me permission to post her description of daily life and symptoms plus her account of the weekend. When I watch my Molly bumble around, I see her very differently.

The following is written by Lynn Burton.

I tried to explain about 'zero Chiari' on Sunday, which means there is no herniation of the brain down nto the spinal cord. But there is overcrowding at the Foramen magnum which means that there is still a blockage of Cerebral Spinal fluid,CSF, which cases pressure. So yes dogs walk on all fours, do not have cerebellar tonsils, but this does not mean CM does not affect them.....it most definitely does. I myself have gently stroked the neck of a CM dog, this lovely dog didn't move his head, wasn't aggressive but gave a low growl to let me me know he wasn't comfortable being touched, I didn't realise at first what this meant but when my hand gently brushed his neck again the same happened. He had been running around, jumping excited to see me in his home, but then as in humans this activity has a price.... pain. His owner settled him with pain medication and he had a good night. But the pain was there , as someone with Chiari I knew exactly why he growled on being touched at the back of his head, there are many days when I feel the same way, although a growl may scare the family!

Dr Clare Rusbridge has shown in video films how dogs with CM are affected by pressure after defecating, running and even barking! How they need to sleep with their heads raised, as to lie flat causes pain in the head. If Chiari affected dogs differently why do dogs show the same symptoms as CM in humans? Balance problems dogs after defacating, barking. Humans after laughing, straining, coughing, leaning forwards, these actions cause horrendous pain as straining etc causes the CSF to 'shoot' from the spinal cord through the Foramen Magnum into the brain, the CSF rushes past the CM or overcrowding but then as the CM or overcrowding is blocking the exit back to the Spinal cord this cause huge CSF pressure build up until the CSF eventuall trickles back through. LIke shaking a bottle of Champagne with the cork in, if the wire has been removed the cork will explode at force from the bottle, however if the wire is left on the pressure is there to see in the form of 'bubbles' the pressure cannot escape but eventually the bubbles subside and settle.
The pain this causes is horrendous, for seconds I cannot move, see, hear just a thumping pressure in the back of the neck and head.

Weather changes also affect head pain low pressure can cause low pressure pain and many fellow patients with Chiari have voiced how the changes in the weather can cause them to feel worse. Heat can also cause pain, in my case a lovely hot sunny day has me reaching for fans. cool drinks anything but overheating as this makes my head feel as though it will explode!
I am in pain every single day, blurred and double vision, tingling and numbness in my arms hands and legs. Loud ringing in my ears. loss of hearing Choking, I dread catching a cold as coughing and sneezing causes tremendous pressure pain. I can't walk far because of the pain in my spine and head, fatigue, the list goes on.

Even in humans doctors do not know a lot about Chiari, every day I receive calls to the charity helpline from patients diagnosed with Chiari but told, " Chiari will just give you a bit of a headache, all your other symptoms are nothing to do with it". but once directed to a surgeons who specialises in Chiari they are usually offered surgery to try to halt the progress, in some cases to avoid paralysis.
Chiari is a rare and complicated condition of the brain and spinal cord which should be taken seriously, in dogs as well as humans. The charity of which I am a trustee, takes CM in dogs so seriously it gave Dr Clare Rusbridge a grant of £6.000 to collect and research DNA from affected dogs to try to find a gene responsible.

I have morphine patches which release morpine into my blood stream 24 hours a day 7 days a week, I also have oral medication for breakthrough pain and while I accept that not all with CM will be as badly affected nearly all will experience some level of discomfort every single day.

I can understand why many of those with Cavaliers will not want to acknowledge that CM can cause such problems in dogs, if CM affects the dogs so adversely where do they go from here?
But if warnings about Syringomyelia/CM had been listened to and acted upon years ago when concerns where first aired and breeding form affected dogs had been stopped maybe this beautiful breed would not be in the dangerous state it is in now. With registrations down by thousands soon it will be impossible to insure these amazing dogs this will put off prospective owners, if the demand for puppies goes down there is a real danger for the breeds survival. Maybe instead of shying away from some kind of 'clear dog ' breeding programme it should be embraced, there is no shame in admitting that 'your' breeding dogs have CM/SM, no shame at all. Shame is when a breeder carries on breeding knowing that their dogs will pass on these horrendously painful conditions.

A lady with Chiari Malformation put the following on our facebook page today in response to a post about breeding from CM/SM affected dogs, it really sums everything up:

"Your comments made me nearly cry. One of our main concerns is can we pass this horrid condition on to our children. The fear of inflicting this pain and torment plays on all our minds. To think that any one would breed an animal knowing they will possibly have Chiari is so sad. I would not wish this on my worst enemy, never mind a dog who can not express their pain.

I hope your good work spreads the word, and people out there listen. Thanks for taking the time to work with the Trust, only positive steps can come out of it."

We know this pain we live with it daily, dogs can't tell you what they go through........we can.

At the talk Lynn gave a candid description of all what she had felt from the intial health problems she had at an early age right through to her current situation. It was harrowing listening to how much her symptoms had been dismissed by the medical profession, how a surgery had been completely bodged up right through to her finally making contact with a doctor that understood CM. After hearing this it has really made me also reconsider my understanding and views of the whole cm / sm scenario. So many people I have heard say that their dog has ONLY got CM. I used to think, well both my dogs have got SM, so you are lucky that yours has only got CM. But now I am thinking maybe they are not so lucky after all as it appears that the pain from CM could be much harder to treat, and the poor dogs that are getting symptoms from both cm and sm goodness knows what level of pain and discomfort they are in.

Thank you so much for passing on all this information for people like me who couldn't be there ladies. I read it through carefully last night and was left in floods of tears, realising what my Rebel must have been going through for so long, but was unable to tell me.

My boy is one of the lucky ones, in that I think his treatment programme has taken away the pain he must have suffered for so long, as he has both conditions. Since adding 300 mgs daily of Gabapentin to his regime Rebel has been a new dog, behaving several years younger than his now 10 years. My previously laid back and reluctant alpha dog now withers younger dogs into corners at every disapproving glance and roars like a lion at anyone, including me, who falls short of his expectations. Frusemide was the wonder drug which dealt with his long standing photophobia.

Thanks for posting this Tania. When Murphy was recently MRI'd and found to have CM, and just a small pre-syrinx I rejoiced at him not being as bad a Misty. Now after his episodes, and his trip to the pain clinic, I realise just how wrong I was to think he "only" has CM.

As much as it grieves me to say it, I will never own another Cavalier, I love my two dearly, and wouldn't swap them for anything, but I couldn't go through this again. Even now I have doubts popping up about them being comfortable. How can we tell if they are really pain free, when they hide their pain so well. Misty has only once "screamed" in pain after jumping off the couch, she must have hurt terribly to have voiced it. This was back when she was first diagnosed, and I never want to hear that sound ever again. She appears happy, she eats well, she likes to go out for walks, she's loving and seeks cuddles, but still how much is she hiding? Has she just learned to tolerate the pain? These thoughts do trouble me, even more so now that Murphy has been diagnosed too. Being on Gabapentin has made Murphy more playful, but he's never shown a reluctance to exercise, and he did still play, just not every night like he used to do, and now does again. They both seem to be happy, but I wish I could be sure. If only they could talk eh?

Thank you so much for sharing this Tania, although it makes devastating reading

I hadn't understood about the reality of living with just CM until Kayleigh was diagnosed, I was expecting a really bad scan with syrinxes due to her severe symptoms and was really shocked when she "only" had CM. As many people will know, she was recently given her wings at 3 years 4 months old, just 15 months after the initial scan. She was under the care of the neurologist at Glasgow vet school together with advice from Clare Rusbridge and a supportive vet. We had exhausted all medical options and surgery had been discounted.

Kayleigh was very stoic and coped incredibly well. It's so hard for us to know what they are really experiencing but when you know your dogs you can see when they are struggling. When I look back to her last day in particular I realise how sore she was, that she was letting us know that she had had enough.

It is helpful to hear from Lynn but just heartbreaking and I am so sorry that she has to live with this horrid condition.

Living with a nerve pain condition myself has given me a perspective on how much these dogs must suffer sometimes.

I don't want to frighten people, we should all enjoy our Cavaliers but think we need to be aware of the problems they can suffer from so we can get help as soon as possible if something happens.

My Oliver has a small syrinx but I have always felt that all his current problems with pain and discomfort stem from his CM, which gives him severely dilated ventricles and resultant pressure behind his eyes, and frequent headaches. He is obviously affected by wide changes in air pressure; will usually only eat if his food is raised to head height, since bending his head to floor level clearly increases pressure; he will greet other dogs when off-lead in the park but has always carefully avoided getting involved in play that might bang his head; he is light phobic to the extent that he will start squinting at sun coming through the window. Many of these symptoms he has had since I had him at a year old, but his CM/SM was only diagnosed in 2007 when he was 6, when after a talk to the Midland Cavalier Club by Geoff Skerritt of Chestergates I took him to the first mini-scan day organised by the Midland Club - not because anyone was telling me he had symptoms but because he had three generations of dogs behind him who had had SM and I wondered if he had it.

His new medication (the result of taking part in the RVC clinical trial earlier this year) is used to reduce inter-cranial pressure in humans (Lynn may well be on it!) and has been very effective in reducing Oliver's headaches. However, he is beginning to air scratch occasionally and show other signs of pain elsewhere, so I wonder if his syrinx is at last on the move. Yet watching him trot around the park in the sunshine this afternoon, stopping to smell interesting scents and wagging his tail at other dogs, I think that he does still enjoy a lot of his life and most of his pain is under control to some extent at least and he can 'switch off' from it when doing what he enjoys. But when he lies beside me on the sofa and paws at my leg because it helps if I stroke his head gently, I weep inside for him and wonder how we ever allowed our beloved Cavaliers to get into this state.

Because of my experience with Oliver, I have always felt that the pain from CM has been underestimated, and welcome the greater interest in CM being shown by researchers. But the implications are very, very depressing. About 90% of Cavaliers have CM - which means that all of them have the potential for severe pain, and many of them may already be suffering it, undiagnosed; and even those that have been MRId may not be correctly treated because they have shown to have no syrinx and therefore they must be 'clear' - clear of SM, but not of CM. And there seems no way out of this situation. An outcross might work - but the gene(s) for CM could have come into the breed (and before them the King Charles Spaniels that provided the ancestors for Cavaliers) many generations ago and take many generations to breed out, even if this is possible at all.

But thanks, Tania, for posting Lynn's talk, which rang so many bells with Oliver's experience and must make everyone who heard or reads it more aware of CM.

Thank you for sharing this information. It upsets me to hear 90% of cavaliers have this condition. I am afraid we don't hear more suffering from it because our dogs are so good about hiding their pain. It makes me sad.

I wonder when (or if) any breeder will be brave enough to start an outcross breeding to correct this fault in our breed. I would love to hear someone making progress in this area. Unfortunately so many are waiting for the medical research to find a way to fix it but clearly we are so far behind. I just don't think purebred dog breeding will be able to go on as we have been doing, we are seeing so many health problems in many breeds that are breed specific. We should remember that many of our lovely purebreds did not start out that way.

One of my favorite breeding programs is the LUA dalmatian project. It really is a tribute to man's love of his breed.

Thankyou for posting this Tania. The health day, and specifically Lynne's talk of living with CM, had quite an impact on me. Having already realised how devastating CM alone can be, through watching my dear little cavalier Maisy struggle with it, I was stunned to realise the extent in which CM has spread throughout the breed. I had assumed that MRI clear cavaliers, meant CM clear, and so had thought that was the way forward, the way of saving this breed. Now I know that practically every cavalier has CM, that this is almost accepted in some circles as normal, and just the way cavaliers heads are now shaped. This left me feeling very depressed, as what does it mean for the future of these amazing little dogs? Lynne had described to us how painful CM alone could be, so if all cavaliers have CM, how much pain are they really in, that we are unaware of? Very thought provoking and unsettling.

Thanks so much for posting this -- this description of CM really does give insight into what the dogs experience and also certainly matches what many of us have observed about our dogs with both CM and SM-- the greater discomfort on days when atmospheric pressure is changing; hot days, etc.

One thing that I would add as a caveat -- is that this isn't unique to CM, as many of the same symptoms are reported by people with SM too. And that CM alone does not produce symptoms in cavaliers at anything close to the rate that SM does; whereas in humans, it is CM that is far more prevalent and tends to produce symptoms regardless of whether it goes on to develop into SM. But there are many cases too of people having the Chiari Malformation but no symptoms - just as with cavaliers.

So it would be wrong to assume that such symptoms would only or even primarily be due to a cavalier's CM alone if a dog also has SM. And it would be wrong to assume many or most dogs with CM likely experience these kinds of severe CM symptoms or even in many cases, any symptoms at all. It is also very wrong for people to dismiss CM on its own as somehow a less serious a problem for many dogs as SM. Clearly numerous people have cavaliers with significant to severe symptoms with CM alone.

As an earlier large study has shown, about a quarter of dogs with SM symptoms had CM alone. That however would suggest that only some dogs with CM experience discomfort from CM alone and a far smaller number than do from SM at some point in their lives because symptomatic SM dogs are a much smaller set of dogs than the general cavalier population to begin with -- yet almost all cavaliers have CM to some degree. There are definitely some differences in the ways human and dogs have this condition, as well --

* the fact that fewer dogs with CM have symptoms, even though almost 100% have CM. Wheras it seems unlikely that many humans live with CM without distinct symptoms eventually, generally showing in their 20s-30s
* the fact that CSF flow is likely to be different due to dogs carrying their heads horizontally
* the lack of cerebellar tonsils in dogs
* the fact that decompression surgery in humans almost always causes a syrinx to collapse or reduce in size, but rarely causes it to collapse or significantly reduce in dogs except with the more invasive mesh surgery (and the fact that this may not make much difference in dogs)
* the fact that most humans with severe CM/SM only really benefit from surgery -- medications don't seem to do much -- and many need to repeat the surgery.

It seems very likely that it is conformation -- the way a cavalier looks, with its shorter muzzle -- that somehow contribute to the incidence of CM. The foetal tissue research project as well as other work on adult dogs indicates this is likely the case. The fact that SM due to CM (and CM) occurs pretty much solely in smaller breeds bred with shorter to flat faces points to nose shortening and the smaller breed size as having some role. Research also indicates that SM in dogs is an inherited genetic condition that likely also has some environmental element (not that 'environmental' is a vast category!). There's also an indication that some cavaliers get a protective gene that keeps CM from developing into SM. So the breeding guidelines and the use of MRIs (especially for OLDER dogs to see whether a given dog is remaining SM-free) is definitely very important for breeding and results with breeders tracking the development of SM in offspring over time show a very significant reduction in incidence and severity when guidelines are followed. There will ALWAYS be exceptions but unfortunately certain breeders constantly cite the exceptions as if they are the rule, whereas they are the exception that helps indicate that the general rules of genetic inheritance apply (high school genetics, folks! ). One really has to ask why these exceptions are dragged up and cited over and over -- unless 1) they give some people an excuse not to bother testing and rely on their 'instinct and breed knowledge' which sure hasn't helped reduce incidence of MVD or SM so far, has it? or 2) they desperately want to discredit researchers whose work inconveniently keeps demonstrating high rates of CM/SM in the breed and the need to MRI and follow breeding guidelines... and MRI older cavaliers rather than rely on a single MRI of a younger dog for a life history of SM.

That said: the researchers and geneticist I have spoken to DO think that there is a very good chance to rescue the breed from the high incidence and higher levels of severity of this condition in cavaliers. BUT that is only if breeders scan, use the EBV programme, follow breeding guidelines, and probably also, reconsider the shape of the cavalier head and perhaps breed size (a larger dog with a longer nose would likely be less likely to have SM, a number of researchers feel). Outcrossing to another breed may be needed but many feel the situation doesn't actually require that IF breeders would actually use the tools they already have and also submit scans for the EBV programme as it will not work with a small number of scans. Yes cost is an issue but so is the survival of the breed. Already some people are not buying cavalier puppies because the genetic probems and their incidence/difficulty in treating are much better known to the general public thanks to programmes like Pedigree Dogs Exposed and websites focused on making information more widely available than what is on most kennel club/breed club sites. Personally I think breeding should be subject to national independent oversight and mandatory testing coupled with a 'lemon law' making breeders (including backyard breeders and puppy farmers) liable for pet owner costs in part or whole if they produce and sell dogs that develop these conditions without having done any testing at all.

CM and SM are very poorly understood in humans -- one expert in human SM that I heard speak, described it as remaining an enigma even after he'd spent his life studying and treating it. Cavaliers are helping human researchers gain new insights into both CM and SM.

I think hearing what humans live with, with these diseases, is extremely important for anyone who owns (and breeds!!!) cavaliers. Hearing from humans gives enormous insight. It is also distressing and disturbing. It remains hard to know however, what dogs experience and to what degree, and it seems every individual case is different with dogs with CM/SM.

Symptoms develop slowly over time, but can come on suddenly after a fall or minor trauma. Sensation may be affected first. Some common symptoms include:

Loss of sensitivity to hot and cold
Numbness and tingling
Bowel and bladder function may be affected
Scoliosis
Pain
Muscle weakness
Spasticity
Paralysis (in severe cases, quadriplegia)
Scoliosis may be the only symptom in children. Often people with scoliosis undergo spinal MR imaging because of an atypical left-sided thoracic curve.

Many individuals suffer from chronic pain and some will develop neuropathic pain syndromes. This type of pain is difficult to treat. A large percentage of people have headaches which can be severe.

Chiari:

The most common symptom of Chiari malformation is a headache, which begins at the back of the head (neck) and radiates upward. The pain is often made worse or can be brought on by coughing, sneezing or straining. These activities are known as valsalva maneuvers.

Visual problems such as nystagmus (involuntary eye movements), double or blurred vision may occur. Balance difficulties, vertigo and dizziness also may be present. Some people may have cranial nerve compression. This can result in apnea (cessation of breathing), gagging, swallowing difficulties, facial numbness or syncope (temporary loss of consciousness).

Patients may have muscle weakness, particularly in the upper extremities, coordination problems, and gait abnormalities. Imaging of the spine may reveal a fluid collection inside of the spinal cord, known as a syrinx. Some individuals may have hydrocephalus, a buildup of fluid in the ventricles of the brain.